Transplant gives Sarah chance to live dreams

Sarah Murnaghan fully understands all she's been through: her lifelong battle with cystic fibrosis that led to her deteriorating lungs, the campaign her parents waged that temporarily changed national policy surrounding organ transplants, and the fact that she came so close to death in the days leading up to her two double lung transplants.

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A 28-year-old woman with cystic fibrosis presented with painful wrinkling of the palms shortly after immersion in water. She had undergone a bilateral single sequential lung transplant five months before. Pertinently, before this, she had been asymptomatic. Her signs and symptoms were consistent with aquagenic wrinking of the palms. Her immunosuppressive regime included tacrolimus 4.5 mg BD, mycophenolate mofetil 500 mg BD, and prednisolone 10 mg OD. Aquagenic wrinking of the palms is a rare condition in which white papules and plaques and excessive wrinkling of the palmar skin occur within minutes of water exposure.

Semin Respir Crit Care Med 2018; 39: 383-391 DOI: 10.1055/s-0038-1651495Nontuberculous mycobacteria (NTM) can cause chronic pulmonary infection in susceptible hosts. Individuals with cystic fibrosis (CF), a multisystem disease predominated by progressive structural lung disease, are particularly vulnerable. Only recently have NTM been recognized for their potential to cause lung deterioration in CF patients. The reported prevalence varies widely from 4 to 40%, significantly more common than in the general population, but this varies because of multiple factors including inconsistent screening practices. Mycobacterium absce...

Authors: Hsin MKY, Wong CF, Yan SW, Fan KY, Ho CKL, Bhatia I, Au TWK
Abstract
Clinical lung transplant was first performed in Hong Kong in 1995. In the early years, the volume of activity was very low. There has been a clear trend of increasing volume in the past few years. The recipient pathology is very different from the International Society for Heart and Lung Transplantation (ISHLT) database, with complete absence of cystic fibrosis and alpha-1-antitrypsin deficiency, and a predominance of diseases of the pulmonary circulation. Lymphangioleiomyomatosis (LAM) has a much higher representation on the waiting list...

A 39-year-old non-smoker woman was admitted in the thoracic oncology unit of the Montpellier Academic Hospital in June 2017 for investigation of multiple lung consolidations, fever, and respiratory failure. She was followed-up since infancy for a cystic fibrosis with cystic fibrosis transmembrane conductance regulator (CFTR) mutation ( ΔF508-CFTR). Two years ago, a lung allograft was decided, owing to rapid onset of respiratory function impairment, and she received a bi-pulmonary transplant. According to the donor database, the lung transplant was removed from a 57-year-old woman with a 30 pack-year smoking history.

Doctors in Scotland believe a woman’s large tattoo on her leg may have been the cause of her unexplained inflammation and severe leg pain.
The authors of a report on the woman’s case, published in BMJ Case Reports, say it is a reminder to physicians that they should be mindful of their patients’ tattoos if they encounter unusual and unexplained symptoms.
The 31-year-old woman was suffering a condition called inflammatory myopathy, which affected her left leg and caused pain severe enough to disrupt her sleep.
The woman had a double lung transplant in 2009, and was taking drugs to suppress her immune syst...

A woman with cystic fibrosis and lung transplants suffered chronic pain for three years after she had a tattoo on her thighGetting a tattoo if you have a weakened immune system could put you at risk of complications, doctors have warned. The caution comes after a woman with cystic fibrosis and lung transplants developed thigh and knee pain after having body art inked on her leg.Doctors say those taking immunosuppressant drugs should take precautions if considering body art. These medicines are often given after an organ transplant or to treat autoimmune conditions such as Crohn ’s disease, lupus or rheumatoid arthrit...

CONCLUSIONS: The lack of currently available evidence makes it impossible to draw conclusions about the comparative efficacy and safety of the various immunosuppressive drugs among people with cystic fibrosis after lung transplantation. A 2013 Cochrane Review comparing tacrolimus with cyclosporine in all lung transplant recipients (not restricted to those with cystic fibrosis) reported no significant difference in mortality and risk of acute rejection. However, tacrolimus use was associated with lower risk of broncholitis obliterans syndrome and arterial hypertension and higher risk of diabetes mellitus. It should be noted...

Over time, cystic fibrosis robbed Nicholas Hemmerle of his ability to enjoy active time with his daughter. But after a double lung transplant, he's breathing easier and ready to have fun again.? The last thing Nicholas Hemmerle remembers before going into surgery at?Mayo Clinic?for a double?lung transplant?is a nurse putting a blanket on him and [...]